Lower and upper motor neuron involvement and their impact on disease prognosis in amyotrophic lateral sclerosis
| العنوان: | Lower and upper motor neuron involvement and their impact on disease prognosis in amyotrophic lateral sclerosis |
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| المؤلفون: | Anna A. Abramova, M N Zakharova |
| المصدر: | Neural Regeneration Research Neural Regeneration Research, Vol 17, Iss 1, Pp 65-73 (2022) |
| بيانات النشر: | Wolters Kluwer - Medknow, 2021. |
| سنة النشر: | 2021 |
| مصطلحات موضوعية: | medicine.medical_specialty, amyotrophic lateral sclerosis, electromyography, motor neuron disease, neuroimaging, Physical examination, Electromyography, Disease, Review, electrodiagnostic medicine, Physical medicine and rehabilitation, Developmental Neuroscience, diagnostic biomarkers, biomarkers of progression, classification, disease heterogeneity, Medicine, Amyotrophic lateral sclerosis, RC346-429, medicine.diagnostic_test, business.industry, Upper motor neuron, Clinical study design, Neurodegeneration, medicine.disease, Clinical trial, medicine.anatomical_structure, Neurology. Diseases of the nervous system, business |
| الوصف: | Amyotrophic lateral sclerosis is a fatal neurodegenerative disease characterized by progressive muscle wasting, breathing and swallowing difficulties resulting in patient’s death in two to five years after disease onset. In amyotrophic lateral sclerosis, both upper and lower motor neurons of the corticospinal tracts are involved in the process of neurodegeneration, accounting for great clinical heterogeneity of the disease. Clinical phenotype has great impact on the pattern and rate of amyotrophic lateral sclerosis progression and overall survival prognosis. Creating more homogenous patient groups in order to study the effects of drug agents on specific manifestations of the disease is a challenging issue in amyotrophic lateral sclerosis clinical trials. Since amyotrophic lateral sclerosis has low incidence rates, conduction of multicenter trials requires certain standardized approaches to disease diagnosis and staging. This review focuses on the current approaches in amyotrophic lateral sclerosis classification and staging system based on clinical examination and additional instrumental methods, highlighting the role of upper and lower motor neuron involvement in different phenotypes of the disease. We demonstrate that both clinical and instrumental findings can be useful in evaluating severity of upper motor neuron and lower motor neuron involvement and predicting the following course of the disease. Addressing disease heterogeneity in amyotrophic lateral sclerosis clinical trials could lead to study designs that will assess drug efficacy in specific patient groups, based on the disease pathophysiology and spatiotemporal pattern. Although clinical evaluation can be a sufficient screening method for dividing amyotrophic lateral sclerosis patients into clinical subgroups, we provide proof that instrumental studies could provide valuable insights in the disease pathology. |
| اللغة: | English |
| تدمد: | 1876-7958 1673-5374 |
| الوصول الحر: | https://explore.openaire.eu/search/publication?articleId=doi_dedup___::d198c8eb043beab6ba868d3e2248a985Test http://europepmc.org/articles/PMC8451581Test |
| حقوق: | OPEN |
| رقم الانضمام: | edsair.doi.dedup.....d198c8eb043beab6ba868d3e2248a985 |
| قاعدة البيانات: | OpenAIRE |
| تدمد: | 18767958 16735374 |
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